Congenital valvar aortic stenosis

Congenital aortic stenosis is characterized by narrowing of the aortic valve orifice, leading to left ventricular hypertrophy and predisposing the patient to exercise intolerance and myocardial dysfunction. Careful monitoring is essential because of the progressive nature of this lesion. Balloon valvuloplasty is the procedure of choice as intermediate palliation for moderate to severe aortic stenosis. If balloon valvuloplasty is contraindicated, surgical valvotomy has a limited role in initial therapy. If balloon valvuloplasty is unsuccessful or contraindicated because of aortic insufficiency, the Ross operation (removal of the stenotic aortic valve and placement of a pulmonary autograft in the aortic position and a pulmonary homograft in the pulmonary position) is the treatment of choice.